Recombinant human GCSH protein
The enzyme system for cleavage of glycine (glycine cleavage system) is composed of four mitochondrial protein components: P protein, H protein, T protein, and L protein. GCSH is the H protein, which transfers the methylamine group of glycine from the P protein to the T protein. Defects in this gene are a cause of nonketotic hyperglycinemia (NKH) . Recombinant human GCSH protein, fused to His-tag at N-terminus, was expressed in E. coli and purified by using conventional chromatography techniques.
Product Specifications
Product Name Alternative
Glycine cleavage system protein H (aminomethyl carrier), GCE, NKH
Expression System
E.coli
Antigen Species
Human
Tag
His-Tag
Applications
SDS-PAGE
Concentration
1 mg/mL (determined by Bradford assay)
Purity
> 95% by SDS-PAGE
Molecular Weight
16.4 kDa (149aa) confirmed by MALDI-TOF
Additionnal Information
References & Citations
Kure S., et al. (2001) J Hum Genet. 46 (7) :378-84.; ; Kovata H., et al. (1991) Am J Hum Genet. 48 (2) :351-61.
Storage Conditions
Can be stored at 2°C to 8°C for 1 week. For long term storage, aliquot and store at -20C to -80C. Avoid repeated freezing and thawing cycles.
Formulation
Liquid in. 20 mM Tris-HCl buffer (pH 8.0) containing 10% glycerol, 1mM DTT, 0.15M NaCl.
Scientific Category
Metabolism
NCBI Accession Number
NP_004474
Uniprot Accession Number
P23434
Species
Human
AA Sequence
SVRKFT EKHEWVTTEN GIGTVGISNF AQEALGDVVY CSLPEVGTKL NKQDEFGALE SVKAASELYS PLSGEVTEIN EALAENPGLV NKSCYEDGWL IKMTLSNPSE LDELMSEEAY EKYIKSIEE
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